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朗格汉斯组织细胞增生症(Langerhans cell histiocytosis,LCH)的皮肤损害较多见,而甲损害则罕见。其临床上可表现为指、趾甲的甲周红斑及肿胀、甲襞破坏、纵向开槽、紫癜样纹、甲下紫癜、甲角化过度、甲下脓疱、甲变形、甲板缺失、甲沟炎、甲剥离、纵形红甲和甲蚀斑。报告1例34岁男性患者,具有尿崩症,突眼,骨损害及皮肤黏膜病变,如:头面部脂溢性皮炎样损害,浸润结节和溃疡斑块,特别是在腋下,外生殖器区域。指甲损害表现为甲周红斑及肿胀、甲分离、甲下紫癜、裂片样出血、纵向开槽及纵形红甲。皮损组织病理检查证实为LCH。成人皮肤弥漫性LCH罕见,误诊率高,确诊应结合组织病理学特征、免疫表型及超微结构检查综合分析,甲损害可能是该病系统病变的征象,但其是否为不良预后的标志却未可知。
Langerhans cell histiocytosis (Langerhans cell histiocytosis, LCH) skin damage is more common, while the damage is rare. Its clinically manifested as refers to the periapical erythema and swelling of the nail, nail damage, longitudinal slotted, purpura-like pattern, purpura under the nail, hyperkeratosis, hypoplasia under armor, nail deformation, lack of deck, a ditch Inflammation, a peel, longitudinal red armor and a plaque. Report A 34-year-old male with diabetes insipidus, exophthalmos, bone lesions and mucocutaneous lesions such as seborrheic dermatitis-like lesions of head and face, infiltrating nodules and ulcerated plaques, especially in the armpits, genitals area. Nail damage manifested as erythema and swelling periwings, a separation, purpura, lobular bleeding, longitudinal slotted and longitudinal red nail. Skin lesions histological examination confirmed as LCH. Diffuse diffuse LCH of adult skin is rare, and the rate of misdiagnosis is high. Diagnosis should be combined with histopathological features, immunophenotypes and ultrastructural examination. Adenocarcinoma may be a sign of systemic lesion, but whether it is a sign of poor prognosis Unknown